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__NOTOC__
 
* The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
===prognosis===
* Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of idovudine and interferon therapy.
 
* Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy.
 
* Adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.
* The table below lists prognostic factors for adult T-cell leukemia patients:<ref name="pmid20425378">{{cite journal| author=Mahieux R, Gessain A| title=Adult T-cell leukemia/lymphoma and HTLV-1. | journal=Curr Hematol Malig Rep | year= 2007 | volume= 2 | issue= 4 | pages= 257-64 | pmid=20425378 | doi=10.1007/s11899-007-0035-x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20425378  }} </ref><ref name="pmid18042693">{{cite journal| author=Matutes E| title=Adult T-cell leukaemia/lymphoma. | journal=J Clin Pathol | year= 2007 | volume= 60 | issue= 12 | pages= 1373-7 | pmid=18042693 | doi=10.1136/jcp.2007.052456 | pmc=PMC2095573 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18042693  }} </ref><ref name="pmid26361794">{{cite journal| author=Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y et al.| title=Treatment and survival among 1594 patients with ATL. | journal=Blood | year= 2015 | volume= 126 | issue= 24 | pages= 2570-7 | pmid=26361794 | doi=10.1182/blood-2015-03-632489 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26361794  }} </ref>
=== Management of Chronic/Smoldering Adult T-cell Leukemia===
 
* Patients may be managed by observation and close follow-up for any symptomatic deterioration. Follow up tests for such patients may include:
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
:* Complete history and physical examination
|valign=top|
:* Serum calcium level
|+
:* Blood urea nitrogen
! style="background: #4479BA; width: 250px; color: #FFFFFF;"|'''Prognostic Factor'''
:* Serum creatinine level
 
:* Serum LDH
! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
:* Chest and abdominal CT scan
 
* Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
|-
:* Topical corticosteroids
 
:* Topical chemotherapy (mechlorethamine)
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Clinical subtype'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* Local radiation (8–36 Gy)
:* Acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good prognosis.
:* Topical retinoids (bexarotene, tazarotene)
|-
:* Phototherapy (UVB, NB-UVB for patch/thin  plaques; PUVA for thicker plaques)
 
:* Topical imiquimod
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Age'''|| style="padding: 5px 5px; background: #F5F5F5;"|
* Zidovudine and interferon combination therapy:
:* Males are associated with a worse prognosis when compared to females.
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
|-
:* Patients who responded to the therapy should be continued on zidovudine and interferon therapy.
 
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
 
* The criteria for complete remission of adult T-cell leukemia patients includes:
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Performance status'''|| style="padding: 5px 5px; background: #F5F5F5;"|
:* Absence of lymphadenopathy
:* Patient's poor [[performance status]] is associated with a worse prognosis.
:* Absence of hepatomegaly and splenomegaly
|-
:* Absence of cutaneous lesions
 
:* Absence of malignant on peripheral blood smear
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Calcium level'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* Absence of malignant on bone marrow biopsy
:* Hypercalcemia is associated with a worse prognosis.
===Management of Acute Adult T-cell Leukemia===
 
* The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:  
|-
:* Cyclophosphamide {{and}} doxorubicin {{and}} vincristine {{and}} prednisone (CHOP)
 
:* Cyclophosphamide {{and}} doxorubicin {{and}}  vincristine {{and}} etoposide {{and}} prednisone (CHOEP)
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Leukocyte count'''|| style="padding: 5px 5px; background: #F5F5F5;" |
:* Etoposide {{and}} prednisone {{and}} vincristine {{and}} cyclophosphamide {{and}} doxorubicin (Dose-adjusted EPOCH)
:* An elevated leukocyte count is associated with a worse prognosis.
:* Cyclophosphamide {{and}} vincristine {{and}} doxorubicin {{and}} dexamethasone (HyperCVAD) alternating with  high-dose methotrexate and cytarabine
 
* Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
|-
* Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
 
:* Bendamustine
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Lactate dehydrogenase (LDH) level'''|| style="padding: 5px 5px; background: #F5F5F5;"|
:* Belinostat
:* Elevated level of [[LDH]] is associated with a worse prognosis.
:* Brentuximab vedotin for systemic CD30+ PTCL 
 
:* DHAP (dexamethasone, cisplatin, cytarabine)
|-
:* ESHAP (etoposide {{and}} methylprednisolone {{and}} cytarabine {{and}} cisplatin)
 
:* Dose-adjusted EPOCH
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''β2-microglobulin level '''|| style="padding: 5px 5px; background: #F5F5F5;"|
:* GDP (gemcitabine {{and}} dexamethasone {{and}} cisplatin)
:* An elevated β2-microglobulin level is associated with a worse prognosis.
:* GemOx (gemcitabine {{and}} oxaliplatin)
 
:* ICE (ifosfamide {{and}} carboplatin {{and}} etoposide)
|-
:* Pralatrexated
 
:* Romidepsin
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Lymphocyte surface markers'''|| style="padding: 5px 5px; background: #F5F5F5;"|
* Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.
:* Over expression of [[CD25]] is associated with a worse prognosis.
* Zidovudine and interferon combination therapy:
|-
:* Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
 
:* Patients who responded to the therapy may be further managed by either allogeneic stem cell transplantion or continue on zidovudine and interferon combination therapy.
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Neuron‐specific enolase'''|| style="padding: 5px 5px; background: #F5F5F5;"|
:* While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
:* An elevated neuron‐specific enolase level is associated with a worse prognosis.
===Management of Adult T-cell Lymphoma===
|}
* The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:  
:* Cyclophosphamide {{and}} doxorubicin {{and}} vincristine {{and}} prednisone (CHOP)
:* Cyclophosphamide {{and}} doxorubicin {{and}}  vincristine {{and}} etoposide {{and}} prednisone (CHOEP)
:* Etoposide {{and}} prednisone {{and}} vincristine {{and}} cyclophosphamide {{and}} doxorubicin (Dose-adjusted EPOCH)
:* Cyclophosphamide {{and}} vincristine {{and}} doxorubicin {{and}} dexamethasone (HyperCVAD) alternating with high-dose methotrexate and cytarabine
* Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
* Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
:* Bendamustine
:* Belinostat
:* Brentuximab vedotin for systemic CD30+ PTCL 
:* DHAP (dexamethasone, cisplatin, cytarabine)
:* ESHAP (etoposide {{and}} methylprednisolone {{and}} cytarabine {{and}} cisplatin)
:* Dose-adjusted EPOCH
:* GDP (gemcitabine {{and}} dexamethasone {{and}} cisplatin)
:* GemOx (gemcitabine {{and}} oxaliplatin)
:* ICE (ifosfamide {{and}} carboplatin {{and}} etoposide)
:* Pralatrexated
:* Romidepsin
* Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.
==Supportive Therapy==
===Opportunistic Infections Prophylaxis===
:* Sulfamethoxazole/trimethoprim prophylaxis is recommended among adult T-cell leukemia patients to protect against opportunistic infections.

Latest revision as of 19:43, 25 January 2016

  • The optimal therapy for adult T-cell leukemia depends on the clinical variant of the disease.
  • Chronic and smoldering adult T-cell leukemia patients are usually managed by either observation, skin directed therapies, or a combination of idovudine and interferon therapy.
  • Acute adult T-cell leukemia patients are usually managed by either chemotherapy, supportive care, allogeneic stem cell transplant, or a combination of zidovudine and interferon therapy.
  • Adult T-cell lymphoma patients are usually managed by either chemotherapy, supportive care, or allogeneic stem cell transplant.

Management of Chronic/Smoldering Adult T-cell Leukemia

  • Patients may be managed by observation and close follow-up for any symptomatic deterioration. Follow up tests for such patients may include:
  • Complete history and physical examination
  • Serum calcium level
  • Blood urea nitrogen
  • Serum creatinine level
  • Serum LDH
  • Chest and abdominal CT scan
  • Skin directed therapies for the management of localized cutaneous lesions among such patients may include:
  • Topical corticosteroids
  • Topical chemotherapy (mechlorethamine)
  • Local radiation (8–36 Gy)
  • Topical retinoids (bexarotene, tazarotene)
  • Phototherapy (UVB, NB-UVB for patch/thin plaques; PUVA for thicker plaques)
  • Topical imiquimod
  • Zidovudine and interferon combination therapy:
  • Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
  • Patients who responded to the therapy should be continued on zidovudine and interferon therapy.
  • While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.
  • The criteria for complete remission of adult T-cell leukemia patients includes:
  • Absence of lymphadenopathy
  • Absence of hepatomegaly and splenomegaly
  • Absence of cutaneous lesions
  • Absence of malignant on peripheral blood smear
  • Absence of malignant on bone marrow biopsy

Management of Acute Adult T-cell Leukemia

  • The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:
  • Cyclophosphamide AND doxorubicin AND vincristine AND prednisone (CHOP)
  • Cyclophosphamide AND doxorubicin AND vincristine AND etoposide AND prednisone (CHOEP)
  • Etoposide AND prednisone AND vincristine AND cyclophosphamide AND doxorubicin (Dose-adjusted EPOCH)
  • Cyclophosphamide AND vincristine AND doxorubicin AND dexamethasone (HyperCVAD) alternating with high-dose methotrexate and cytarabine
  • Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
  • Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
  • Bendamustine
  • Belinostat
  • Brentuximab vedotin for systemic CD30+ PTCL
  • DHAP (dexamethasone, cisplatin, cytarabine)
  • ESHAP (etoposide AND methylprednisolone AND cytarabine AND cisplatin)
  • Dose-adjusted EPOCH
  • GDP (gemcitabine AND dexamethasone AND cisplatin)
  • GemOx (gemcitabine AND oxaliplatin)
  • ICE (ifosfamide AND carboplatin AND etoposide)
  • Pralatrexated
  • Romidepsin
  • Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.
  • Zidovudine and interferon combination therapy:
  • Chronic/smoldering adult T-cell leukemia patients should be evaluated for response after two months of initiating the combination therapy.
  • Patients who responded to the therapy may be further managed by either allogeneic stem cell transplantion or continue on zidovudine and interferon combination therapy.
  • While patients who did not respond to the therapy should be managed by either chemotherapy or supportive care depending on the patients preference.

Management of Adult T-cell Lymphoma

  • The first line chemotherapeutic regimens used for the initial management of adult T-cell leukemia include:
  • Cyclophosphamide AND doxorubicin AND vincristine AND prednisone (CHOP)
  • Cyclophosphamide AND doxorubicin AND vincristine AND etoposide AND prednisone (CHOEP)
  • Etoposide AND prednisone AND vincristine AND cyclophosphamide AND doxorubicin (Dose-adjusted EPOCH)
  • Cyclophosphamide AND vincristine AND doxorubicin AND dexamethasone (HyperCVAD) alternating with high-dose methotrexate and cytarabine
  • Consider allogeneic stem cell transplantion for patients who respond to first line chemotherapeutic agents.
  • Patients who do not respond to the initial chemotherapeutic regimens may be managed by other second line chemotherapeutic agents such as:
  • Bendamustine
  • Belinostat
  • Brentuximab vedotin for systemic CD30+ PTCL
  • DHAP (dexamethasone, cisplatin, cytarabine)
  • ESHAP (etoposide AND methylprednisolone AND cytarabine AND cisplatin)
  • Dose-adjusted EPOCH
  • GDP (gemcitabine AND dexamethasone AND cisplatin)
  • GemOx (gemcitabine AND oxaliplatin)
  • ICE (ifosfamide AND carboplatin AND etoposide)
  • Pralatrexated
  • Romidepsin
  • Consider allogeneic stem cell transplantion for patients who respond to second line chemotherapeutic agents.

Supportive Therapy

Opportunistic Infections Prophylaxis

  • Sulfamethoxazole/trimethoprim prophylaxis is recommended among adult T-cell leukemia patients to protect against opportunistic infections.